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- Title
KBG Syndrome: Clinical Features and Specific Dental Findings.
- Authors
Almandey, Abdul Hakim; Anthonappa, Robert P.; King, Nigel M.; Cheuk-wing Fung
- Abstract
Purpose: This investigation sought to identify the common manifestations of KBG syndrome in the literature and to determine the major oral abnormalities in a 12-year-old Chinese boy. Methods: PUBMED search, using the keyword phrase "KBG syndrome," produced 20 articles. Due to the disparities in the quality of evaluations in the reports, any physical feature that was not discussed was assumed to be absent. Results: Of the 54 cases, including the present case, 36 (67%) were moles. Mental retardation or global developmental delay was reported in 42 (78%) cases, while 46 (85%) exhibited shortness of stature. Oral features occurred in 53 (98%) cases; macrodontia was present in 49 (96%) cases; the present case was the only one with hyperdontia and a talon cusp. Other features were croniofacial anomalies, followed by abnormalities of the nose, hand, mouth, eyes, eyebrows, philtrum, costovertebrae, ears, and, less frequently, low hairline and lower extremity abnormalities. Conclusions: Cardinal features of KBG syndrome include facial dysmorphism, short stature, skeletal anomalies, and mild developmental delay; intraorally, macrodontia of the maxillary central incisors occurs in most cases. This is the first known report of KBG syndrome in a Chinese subject and the only case with hyperdontia and a talon cusp.
- Subjects
SUPERNUMERARY teeth; INCISORS; CHILD patients; INTELLECTUAL disabilities; SHORT stature; FACIAL abnormalities; SKELETAL abnormalities
- Publication
Pediatric Dentistry, 2010, Vol 32, Issue 5, p439
- ISSN
0164-1263
- Publication type
Case Study